Lymphadenopathy driven by TCR-Vγ8Vδ1 T-cell expansion in FAS-related autoimmune lymphoproliferative syndrome

Author:

Vavassori Stefano1,Galson Jacob D.12,Trück Johannes1,van den Berg Anke3,Tamminga Rienk Y. J.4,Magerus-Chatinet Aude5,Pellé Olivier5,Camenisch Gross Ulrike6,Marques Maggio Ewerton6,Prader Seraina1,Opitz Lennart17,Nüesch Ursina1,Mauracher Andrea1,Volkmer Benjamin1,Speer Oliver1,Suda Luzia8,Röthlisberger Benno8,Zimmermann Dieter Robert6,Müller Rouven9,Diepstra Arjan3,Visser Lydia3,Haralambieva Eugenia5,Neven Bénédicte5,Rieux-Laucat Frédéric5,Pachlopnik Schmid Jana110

Affiliation:

1. Pediatric Immunology and Children’s Research Center, University Children's Hospital Zurich, Zurich, Switzerland;

2. Vaccine Group, University of Oxford, Oxford, United Kingdom;

3. Department of Pathology and Medical Biology and

4. Paediatric Hematology and Oncology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands;

5. INSERM unité mixte de recherche 1163, Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Paris, France;

6. Department of Pathology, University Hospital Zurich, Zurich, Switzerland;

7. Functional Genomics Center Zurich, Swiss Federal Institute of Technology Zurich, University of Zurich, Zurich, Switzerland;

8. Medical Genetics, Institut für Labormedizin, Kantonsspital Aarau, Aarau, Switzerland;

9. Division of Hematology, University Hospital Zurich, Zurich, Switzerland; and

10. Medical Faculty, University of Zurich, Zurich, Switzerland

Abstract

Key Points FAS-dependent apoptosis in Vδ1 T cells makes the latter possible culprits for the lymphadenopathy observed in patients with FAS mutations. Rapamycin and methylprednisolone resistance should prompt clinicians to look for Vδ1 T cell proliferation in ALPS-FAS patients.

Publisher

American Society of Hematology

Subject

Hematology

Cited by 4 articles. 订阅此论文施引文献 订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献

1. The Proportion of Vδ1T Cells in Peripheral Blood Is Correlated with Prognosis of Sepsis;IRAN J IMMUNOL;2022

2. Incidental splenomegaly in a teenager;Archives of disease in childhood - Education & practice edition;2022-08-08

3. Autoimmune lymphoproliferative syndrome;Stiehm's Immune Deficiencies;2020

4. FAS and RAS related Apoptosis defects: From autoimmunity to leukemia;Immunological Reviews;2018-12-18

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